Marfan Syndrome and Related Heritable Thoracic Aortic Aneurysms and Dissections

Author(s): Julie De Backer , Marjolijn Renard , Laurence Campens , Laura Muino Mosquera , Anne De Paepe , Paul Coucke , Bert Callewaert , Yskert von Kodolitsch .

Journal Name: Current Pharmaceutical Design

Volume 21 , Issue 28 , 2015

Abstract:

In this overview we aim to address a number of recent insights and developments regarding clinical aspects, etiology, and treatment of Heritable Thoracic Aortic Disease (H-TAD). We will focus on monogenetic disorders related to aortic aneurysms. H-TADs are rare but they provide a unique basis for the study of underlying pathogenetic pathways in the complex disease process of aneurysm formation. The understanding of pathomechanisms may help us to identify medical treatment targets to improve prognosis.

Among the monogenetic aneurysm disorders, Marfan syndrome is considered as a paradigm entity and many insights are derived from the study of clinical, genetic and animal models for Marfan syndrome. We will therefore first provide a detailed overview of the various aspects of Marfan syndrome after which we will give an overview of related H-TAD entities.

Keywords: Marfan syndrome, aortic aneurysm, connective tissue disorders, heritable thoracic aortic disorders.

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Article Details

VOLUME: 21
ISSUE: 28
Year: 2015
Page: [4061 - 4075]
Pages: 15
DOI: 10.2174/1381612821666150826093152
Price: $58

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